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[Childhood acromelalgia a propos of a case revealing Fabry's disease].

The occurrence of an acrosyndrome (Raynaud's phenomenon, erythermalgia, acrodynia...) in childhood may be the first manifestation of a general disease. Though it can be an early onset Raynaud's disease, it could also be the first sign of a connective tissue disease (juvenile polyarthritis, mixed connectivitis...) or of a overload disorder. We report a case of childhood-onset acromelalgia leading to the discovery of Fabry's disease. This chromosome X-linked hereditary disorder, resulting in the ubiquitous accumulation of neutral sphingolipids, is usually rapidly suspected by the finding of "boxer-short" angiokeratoma. Diagnosis is confirmed by the ophthalmic examination (cornea verticillata), by the pathological examination of a skin sample, and by the measure of alpha-galactosidase A activity. Treatment is usually only symptomatic, but the discovery of the mutations responsible for the disease could open the way to specific therapy.

Adult↗

[A great Franco-Mauritian epidemiologist: Joseph Désiré Tholozan (1820-18970].

Born in 1820 from French parents in Diego Garcia, an islet then linked to Mauritius where he started in Port-Louis his school years, Joseph Désiré Tholozan was an original personality. He undertook medical studies in France (M. D. thesis, Paris, 1843) after having joined the military Health Service (1841) as a surgeon serving in various garrisons in the country and later at the Hospital of the Valde-Grâce in Paris (1849). Successful at the "agrégation" of Medicine in 1853, he later participated to the Crimean War (1854-1855) where he performed-interesting medical observations. In 1858, he was appointed personal physician to Nasreddin Shah and remained in Persia until his death in Teheran (1897) where he is buried. Tholozan published between 1847 and 1892 over fifty articles and books dealing chiefly with infectious pathology and epidemiology, written at a time when microbial etiology and specificity of such diseases were wholly unknown. He considered chiefly bubonic plague, studying as soon as 1871 the focus of the Iranian Kurdistan, a research which will be resumed by M. Baltazard and his collaborators between 1947 and 1971, i.e. a century later. He was also deeply interested by the "oriental" cholera of which he recalled masterly the history and geography in the Near and Middle East. He also performed, while in Crimea and Persia, personal observations on tuberculosis, diptheria, remittent fever, acrodynia and had studied in France in his early years various other diseases such as cutaneous staphylococcic infections, glanders, pulmonary haemorrhagies, etc. In Persia, he reorganized Public Health and medical teaching and educated many local physicians and surgeons. Being assured of the unlimited confidence of the Shah, he played an important cultural role, promoting French influence in Persia. Holder of many French and foreign decorations, Tholozan was Fellow of the French Academies of Sciences and Medicine. His name was given by Laboulbène to Ornithodoros tholozani, a tick vector of a recurrent fever (spirochetosis due to Borrelia persica), of which he had described both the symptoms and the vector in 1882.

Epidemiology↗

Pink disease.

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Acrodynia↗